Background: In this case report, we describe an unusual case of a patient with myoclonus only occurring during menses. Case Report: A 41-year-old female, known to have neurological sequelae after a car accident 1 year earlier, presented with myoclonic movements of the right arm and hand only during menses. Brain magnetic resonance imaging is compatible with head trauma. Electromyography shows b...

Among paraneoplastic neurologic disorders (PND), opsoclonus-myoclonus syndrome, so-called "dancing eye syndrome," is a rare disorder combining multivectorial eye movements, involuntary multifocal myoclonus, and cerebellar ataxia. Although several paraneoplastic antibodies against postsynaptic or cell-surface antigens have been reported, usually most patients are serum antibody negative. We repo...

Progressive supranuclear palsy (PSP), a neurodegenerative disease with the parkinsonism, is characterized by supranuclear eye movement disorders and static reflex disorder appearing in the neck / trunk from early period of muscle rigidity and illness. Myoclonus is the lightning involuntary muscle shrinkage resulting from excessive neurologic excitement. This disease develops with various condit...

The origin of myoclonus in patients with complex regional pain syndrome (CRPS) is unknown. Eight patients with CRPS related myoclonus were clinically evaluated and studied with intermuscular and corticomuscular coherence analysis. Jerks were present at rest, aggravated during action and were frequently associated with tremulousness or dystonia. Electromyography demonstrated a burst duration ran...

Four patients with cortical myoclonus were studied. All had reflex muscle jerking and grossly enlarged somatosensory evoked responses (SEPs) following electrical stimulation of the digital nerves. In addition, three of the patients had spontaneous or action-induced myoclonus. Back-averaging the EEG from these spontaneous muscle jerks showed a large positive wave over the contralateral somatomot...

BACKGROUND Niemann-Pick type C (NP-C) is a rare autosomal recessive progressive neurodegenerative disorder caused by mutations in the NP-C 1 or 2 gene. Besides visceral symptoms, presentation in adolescent and adult onset variants is often with neurological symptoms. The most frequently reported presenting symptoms of NP-C in adulthood are psychiatric symptoms (38 %), cognitive decline (23 %) a...

objective: hashimoto encephalopathy (he) is known as a steroid-responsive encephalopathy associated with autoimmune thyroiditis or nonvascular inflammation-related autoimmune meningoencephalitis. the average age of onset of he is approximately 50 years; and it is more common in women. the onset of he may be acute or subacute. the course of most he cases is relapsing and remitting, which is simi...

The phenotypic spectrum of STXBP1-related encephalopathy ranges from infantile epileptic to intellectual disability with nonsyndromic or absent epilepsy. Although being frequently reported, the tremor associated STXBP1 has not been fully characterized date. aim our study was describe it. We recruited patients due variants, regardless their phenotype, who had at examination and underwent neuroph...

A 20-year-old woman with subacute sclerosing panencephalitis, bedridden because of dystonic posturing and very frequent myoclonic involuntary movements, improved dramatically with carbamazepine. The favorable effect of the carbamazepine on myoclonus was demonstrated by discontinuing and reintroducing carbamazepine, and videoing the consequent reappearance and disappearance of the myoclonus. [Pu...

In a case of acute disseminated encephalomyelitis displaying opsoclonus-myoclonus syndrome, magnetic resonance images (MRI) disclosed pontine lesions involving part of the pontine paramedian reticular formation and the raphe nucleus. Shrinkage of the lesions correlated with the patient's clinical state. This observation supports the hypothesis that a pontine lesion can cause opsoclonus-myoclonu...